Aetiology

As is the case with other autoimmune diseases, the exact pathophysiological mechanism that causes immune dysregulation in pemphigus is unknown. Pemphigus is a T-cell driven autoantibody-mediated disease, with T cells reacting to specific antigenic adhesion proteins in the skin. The production of autoantibodies to intercellular proteins in the epidermis, along with various inflammatory mediators, is believed to directly elicit acantholysis. The clinical phenotype of pemphigus is defined by the specific profile of intercellular autoantibodies. Patients with mucosal-dominant pemphigus vulgaris have only antidesmoglein 3 IgG autoantibodies. Patients with mucocutaneous pemphigus vulgaris have both antidesmoglein 3 and antidesmoglein 1 autoantibodies. Patients with pemphigus foliaceus, who have only skin lesions, have antidesmoglein 1 antibodies.1

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